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Dedifferentiation rescues senescence of progeria cells but only while pluripotent

Niedernhofer, LJ and Glorioso, JC and Robbins, PD (2011) Dedifferentiation rescues senescence of progeria cells but only while pluripotent. Stem Cell Research and Therapy, 2 (3).

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Abstract

Hutchinson-Gilford progeria syndrome (HGPS) is a genetic disease in which children develop pathologies associated with old age. HGPS is caused by a mutation in the LMNA gene, resulting in the formation of a dominant negative form of the intermediate filament, nuclear structural protein lamin A, termed progerin. Expression of progerin alters the nuclear architecture and heterochromatin, affecting cell cycle progression and genomic stability. Two groups recently reported the successful generation and characterization of induced pluripotent stem cells (iPSCs) from HGPS fibroblasts. Remarkably, progerin expression and senescence phenotypes are lost in iPSCs but not in differentiated progeny. These new HGPS iPSCs are valuable for characterizing the role of progerin in driving HGPS and aging and for screening therapeutic strategies to prevent or delay cell senescence. © 2011 BioMed Central Ltd.


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Details

Item Type: Article
Status: Published
Creators/Authors:
CreatorsEmailPitt UsernameORCID
Niedernhofer, LJniedernh@pitt.eduNIEDERNH
Glorioso, JCglorioso@pitt.eduGLORIOSO
Robbins, PDprobb@pitt.eduPROBB
Centers: Other Centers, Institutes, Offices, or Units > Pittsburgh Cancer Institute
Date: 9 September 2011
Date Type: Publication
Journal or Publication Title: Stem Cell Research and Therapy
Volume: 2
Number: 3
DOI or Unique Handle: 10.1186/scrt69
Schools and Programs: School of Medicine > Microbiology and Molecular Genetics
Refereed: Yes
Article Type: Review
Date Deposited: 27 Oct 2016 19:30
Last Modified: 02 Feb 2019 16:58
URI: http://d-scholarship.pitt.edu/id/eprint/30053

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